Australian X disease See Murray Valley encephalitis
A. Fulfils criteria for migraine with aura
1. The Russian alternative to the Babinski sign, described independently but in a language less conducive to
in-ternational understanding
2. Paradoxical dilation of a pupil with light stimulation in syphilis
3. Plantar flexion of the foot when the dorsum is tapped with a percussion hammer, seen in pyramidal tract dis-ease
4. In pyramidal disease of the cord and in hemiplegia:
“where the foot is passively bent in a plantar direction, when released, dorsal flexion of the foot occurs with flexion of the knee and the hip on the opposite side.”4723 5. (hypogastric reflex) Normal contraction of the muscles of the lower part of the abdominal wall when the thigh is scratched
6. Lack of pain when the peroneal nerve is compressed, seen in tertiary neurosyphilis. This is the same as the Biernacki sign, but in a different nerve. It also resem-bles the Abadie sign.
7. (nasal reflex) Contraction of the facial muscles on the same side when the nose is tickled. This is a normal phe-nomenon.
8. When the patient with severe sciatic nerve irritation ex-tends the sound leg, the affected leg cannot be extended at all.
9. (arm dropping test) When the arms are supported with the shoulders and elbows flexed and the support is sud-denly removed, they will fall to the side after a momen-tary delay in normal subjects, but will “hang” in the air for a second or so in patients with pyramidal disease.
10. Extension and adduction of the thumb in response to a gentle pressure sliding down the border of the radius.
This is a sign of pyramidal tract disease.1349a Bekhterev, Vladimir Mikhailovich von (1857–
1927) Russian neuropathologist and psychiatrist at
Kazan and later in Leningrad. He trained with Fleschig, Meynert, Westphal, and Charcot and authored over 500 papers on anatomy, the vestibular system, cortical localization, and psychology. He described spondylitis deformans (Bekhterev-Marie-Strumpell disease) as well as the conditions defined in the following entries. He maintained poor relations with Pavlov, his colleague at Leningrad, who did not consider his work to be of much merit.1829
Bekhterev-Brissaud syndrome Spasmodic laughter and weeping in patients with hemiplegia; probably the first account of pathological emotionality, since in-volvement of the brainstem was suggested by the orig-inal papers of Brissaud.435,758
Bekhterev-Mendel reflex Elicitation of the plantar muscle reflex by tapping on the dorsum of the foot, thus depressing it, lengthening the arch, and stretching the plantar muscles. This in turn leads to plantar flex-ion of the toes, with dorsiflexflex-ion being the normal re-sponse.
Belgian Society of Clinical Neurophysiology A pro-fessional organization. Contact: Professor J.E. Desmedt, Université Libre de Bruxelles, Unité de Récherche sur le cerveau, 115 bd de Waterloo B-1000, Bruxelles, Bel-gium.
Bell, Luther Nineteenth-century American psychia-trist and superintendent of an asylum in Somerville, Massachusetts.
Bell mania Acute delirium.
Bell-Magendie law The anterior spinal roots are mo-tor, the posterior roots sensory.
The name of Sir Charles Bell should not really join that of Magendie here, because no such conclusion was drawn by him until after Magendie had published his account.
Bell palsy (facial palsy, refrigeration palsy, rheumatic palsy) Facial paralysis due to a lesion of the seventh cra-nial nerve peripheral to the brainstem and commonly in the facial canal, where viral infection (HSV) is con-sidered one likely cause.
Pain in the mastoid region may be the first symp-tom, after which the face is weak, as shown by inabil-ity to wrinkle the forehead or to close the eye on that side, tearing, dysarthria due to drooping of the corner of the mouth, and loss of the nasolabial fold. The sense of taste may be lost over the anterior two-thirds of the tongue. Recovery occurs within 6 months in over 80%
of patients.438
The alternative name reflects the long-held but un-proven belief that cooling of the face may precipitate the palsy, as Bell himself noted in one case. Melotte3823 indicates that an account of the palsy was given by Richard Powell 8 years before Bell’s classic description.
Recurrent facial palsy may in some instances be famil-ial when it carries a worse prognosis for recovery.93
BELL PALSY 99
A variant form is familial recurrent Bell palsy with oc-ular motor palsies, a dominantly inherited syndrome with the first episode of facial weakness occurring at any age and of the first episode of oculomotor paresis (with pupillary sparing) occurring in mature adult life.
The pathogenesis is unknown.93
Bell phenomenon (palpebro-oculogyric reflex) The normal tendency of the eyes to roll upward when a per-son tries to close them. In the presence of facial palsy, with resulting paralysis of the orbicularis oculi, this de-viation, normally hidden, is visible, since the eye is no longer covered by the eyelid. Bell described it in 1823.439 Bell, Sir Charles (1774–1842) Scottish anatomist, surgeon, and artist who went to London in 1804, re-turning as professor of surgery at the University of Ed-inburgh in 1835. Many of his discoveries were enabled in part by his attendance on the wounded after the bat-tles of Corunna and Waterloo. He was a man of great good humor, acknowledged as the leading anatomist of his day. He described facial palsy in 1821, and later re-ported myotonia and pseudohypertrophic muscular dystrophy. He determined that muscles only twitched when the anterior spinal roots were sectioned, but thought that these roots carried both motor and sen-sory impulses. He was aware that posterior root section produced loss of pain but thought that the pathway for unconscious impressions (position sense) was through the posterior roots to the cerebellum; it was Magendie who showed their general sensory function, in 1822, a view that Bell accepted fully.
Bell also noted that the trigeminal nerve has both mo-tor and sensory functions. He identified the long tho-racic nerve,438and was the first to postulate the existence of a “sixth” sense, that of movement in muscles or joints;
this and many other of his anatomical findings were pub-lished in his books The Anatomy of the Body (1802) or The Nervous System of the Human Body (1830).
His engaging personality prompted the text of his epitaph in Hallow Churchyard, Worcestershire:
Sacred to the Memory of Sir Charles Bell who, after Unfolding with Unrivalled Sagacity, Patience and Success
the Wonderful Structure of our Mortal Bodies, Esteemed Lightly of his Greatest Discoveries
Except only as they Tended to Impress Himself and Others with a Deeper Sense
of
The Infinite Wisdom and Ineffable Goodness of the
Almighty Creator.
belly-dancers’ dystonia Focal dyskinetic movements affecting the abdominal wall musculature, most often as an unwanted effect of metoclopramide. Forward thrusting of the pelvis is another such movement sim-ilarly occasioned.2730
Bender Gestalt Test A test of visuospatial praxis in which the subject is required to copy designs shown for a brief period.447The results allow comment upon right hemisphere and frontal lobe functions.
Bender, Morris B. (d. 1983) American neurologist at Mount Sinai Hospital, New York. His major area of in-terest was neuro-ophthalmology and the higher func-tions associated with vision. A superb intuitive clinician and teacher, he was rewarded with a personal Chair in Clinical Neurology and was at one time president of the American Neurological Association.
Bender-type myopathy See congenital myopathy.
bending reflex Flexion of the elbow and shoulder when the wrist is forcibly passively flexed; it is present in normal subjects but is markedly increased with bifrontal lesions, especially those affecting the parasagittal, precentral regions. In severe cases, the el-bow flexion can be elicited by gentle radial deviation of the patient’s wrist (radial bending reflex). The reflexes of Leri and of Mayer are related.5083
bending-over vertigo The physiological occurrence of vertigo or postural imbalance induced when a sub-ject bends maximally at the waist, caused by the same mechanism as head-extension vertigo with the addi-tion of increased intracranial pressure.727
bends See decompression sickness.
Benedek klazomania (Gr, klazo, I howl) Compulsive palilalic shouting in postencephalitic patients, differen-tiated by Benedek from the encephalitic complications of rage attacks, somnambulism, delirium, chorea, and parkinsonism, the latter having been present in all of his postencephalitic patients.453
Benedek, Laszlo (b. 1887) Hungarian. Professor of Neuropsychiatry at Debrecen University and later at Budapest.
benediction hand The appearance of the hand when trying to make a fist when there is a high lesion of the median nerve, preventing flexion of the index and mid-dle fingers because of paresis of the flexor digitorum sublimis and of the median half of the flexor digitorum profundus.
Benedikt inferior syndrome A pontine lesion, usu-ally vascular, causing contralateral hemiplegia, hemi-ataxia, and sensory change.4723
Benedikt, Moritz (1835–1920) Austrian physician who practiced in Vienna. His interest in cerebral local-ization led him to embrace phrenology, in the course of which studies he was one of the first physicians to X-ray the skull. His description of the syndrome now known by his name (1889) was brief and somewhat dis-torted by the facts that his 4-year-old patient also had a left cerebellar and many frontal tuberculomas, a right cingulate gyrus lesion and another in the cerebral pe-100 BELL PHENOMENON
duncle, and syphilis; but his intuitive localization of the lesion responsible to the midbrain was nevertheless cor-rect.454
He was also a pioneer in electrotherapy and in the study of the occupational neuroses.
Influenza A and B, parainfluenza virus, and aden-ovirus 2 are the commoner causal agents. See also postinfluenzal myositis.
benign age and localization-related idiopathic epilepsy See benign childhood epilepsy with cen-trotemporal spikes, benign childhood epilepsy with occipital paroxysms.
benign cerebral vasculitis An inflammatory disor-der of the intracranial arteries associated with segmen-tal narrowing seen on angiography. The condition may be asymptomatic or seizures and focal neurological signs may result. The reversibility of the arteriographic findings and the absence of biopsy reports allow spec-ulation that vasospasm (rather than vasculitis) is the cause in at least some cases.5068
benign childhood epilepsy with affective symptoms A disputed epileptic syndrome, in which frequent, brief periods of extreme fear occur in young children, with-out alteration in consciousness but with the usual au-tonomic symptoms of extreme anxiety. No consistent EEG abnormality is associated.
The condition appears to be self-limiting within a year or two, clearing without residua.1251,4317
benign childhood epilepsy with centrotemporal spikes (rolandic seizures, sylvian epilepsy, benign epilepsy, benign sylvian seizures with midtemporal spike foci, benign childhood epilepsy with rolandic paroxys-mal discharges, lingual syndrome, centrotemporal epilepsy; OMIM 117100) A common form of `benign age and localization-related idiopathic epilepsy characterized by brief, simple, partial, hemifacial motor seizures, fre-quently having associated somatosensory symptoms that have a tendency to evolve into generalized tonic-clonic seizures. Both seizure types are often related to sleep. The onset of these attacks is between the ages of 3 and 13 years (peak: 9–10 years), and recovery occurs sponta-neously before the age of 16 years. Genetic predisposi-tion is frequent (linked to chromosome 15q14), and there is male predominance.3464
The EEG shows blunt, high-voltage centrotemporal spikes, often followed by slow waves that are activated by sleep and tend to spread or shift from side to side.
(Adapted from Commission for classification and ter-minology, ILAE. Proposal for Revised Classifications of Epilepsies and Epileptic syndromes. Epilepsia 1989;
30:389–399.)1114
In brief, it is a rare example of a distinct syndrome of epilepsy, occurring mainly during sleep in prepu-bertal children who are without neurological or intel-lectual abnormalities. The seizures are usually brief and infrequent, and the whole disorder only occupies a few years of the child’s life.56The seizures tend to be con-trollable without great difficulty, and after their termi-nation, the child is left without physical or psycholog-ical handicap.3355
BENIGN CHILDHOOD EPILEPSY WITH CENTROTEMPORAL SPIKES 101
Moritz Benedikt
Benedikt syndrome (tegmental syndrome) The as-sociation of a third cranial nerve palsy with ipsilateral cerebellar tremor and contralateral rubral tremor, in-voluntary movements resembling parkinsonian tremor, and hemiparesis, usually as a result of a small infarct or tumor in the region of the red nucleus which damages both this and the adjacent corticospinal fibers in the cerebral peduncle.456Benedikt actually described it in a patient with a tuberculoma at that site.
In this syndrome, motor fibers cross to the red nu-cleus from the contralateral dentate nunu-cleus, and then cross back to influence the performance of motor ac-tions on the other side of the body, while the corti-cospinal fibers are also uncrossed at this level, resulting in the unusual association of both cerebellar-like and pyramidal findings on the same side.454
benign abducens nerve palsy of childhood A rare, self-limiting condition seen in infancy and childhood.
The diagnosis is essentially one of exclusion.1081 benign acute childhood myositis (myalgia cruris epidemica) An acute infectious syndrome of children causing muscle inflammation, after which myalgia in-volves the calves and thighs more frequently than other muscles; mild weakness and local tenderness are pres-ent and serum creatine kinase levels are markedly in-creased.187Recovery usually occurs within a week.
Despite the usual eponym, given above, the syn-drome was actually first described by Martinus Rulando in 1597.5733
Variant forms include one in which the condition is dominantly inherited and complicated by speech dys-praxia,4946 and malignant rolandic-sylvian epilepsy, a form characterized by refractory sensorimotor seizures, cognitive disturbances, normal MRI findings, perisyl-vian spikes on magnetoencephalography, and fron-totemporal spikes on the EEG.4275
benign childhood epilepsy with frontal or with mid-line spikes A syndrome with clinical features resem-bling those of benign childhood epilepsy with cen-trotemporal spikes in which, however, the spikes are recorded from these differing locations.
benign childhood epilepsy with occipital paroxysms A form of benign age and localization-related idio-pathic epilepsy occurring before the age of 13 years and characterized by unequivocal clinical evidence of epileptic seizures (mainly tonic deviation of the eyes and vomiting, often with evolution to generalized tonic-clonic or to simple motor seizures) and with paroxysms maximal in the occipital areas on at least one EEG, and neither clinical nor EEG manifestations of primary generalized epilepsy nor of any other dis-tinctive syndrome; no evidence of neurological or in-tellectual deficit; and a normal appearance of the CT brain scan.2053,4316
benign childhood epilepsy with rolandic paroxysmal discharges See benign childhood epilepsy with cen-trotemporal spikes.
benign childhood epilepsy with somatosensory-evoked spikes An epileptic syndrome of children in the first decade, characterized clinically by daytime ver-sive seizures affecting the head and body, not always with alteration in consciousness, and electrically by sharp- and slow-wave complexes recorded in parietal or parasagittal regions, elicited by somatosensory stim-ulation, such as tapping the limbs. The attacks tend to remit after a year or so.4317
benign childhood partial epilepsies A group of syn-dromes characterized by the occurrence of partial epileptic seizures in young children, occurring over the space of a few years only, usually at night, and often ac-companied by hypersalivation, vomiting, headache, pal-lor, and sweating. Abundant high-amplitude, focal, sharp- and slow-wave discharges are recorded interic-tally on the EEG from various cephalic regions, espe-cially during sleep, but all other tests give normal re-sults. Febrile convulsions are common in these children.
The syndromes delineated to date include benign childhood epilepsy with affective symptoms; benign childhood epilepsy with centrotemporal spikes;
Panayiotopoulos syndrome; benign childhood epilepsy with occipital paroxysms; benign childhood epilepsy
with somatosensory evoked spikes; and benign child-hood epilepsy with frontal or with midline spikes.4317 See also Landau-Kleffner syndrome.
benign congenital hypotonia A syndrome diag-nosed on the basis of a clinical impression that an in-fant shows more hypotonia than weakness. This is un-likely to be a single entity and some of the conditions are probably not even myopathic. See benign congen-ital myopathy.
benign congenital myopathy (Oppenheim disease, myotonia, amyotonia congenita, benign congenital hy-potonia, minimal change myopathy, floppy infant syn-drome) A heterogeneous group of conditions affecting infants in which hypotonia, muscle weakness, and lax (hypermobile) joints but normal muscle biopsy, serum enzymes, and EMG findings are the major features.1744 The subjects correctly diagnosed in retrospect are those that recover after the first year of life. Others (ob-viously not suffering from a benign disease) die of res-piratory complications of their weakness4250,5669,5837and should be classified as having some other condition.
When combined with hyperexcitability of the CNS, the condition has been known as Ullrich syndrome.2764,5838 benign congenital myotonia See benign hereditary myopathy.
benign cough headache Headache precipitated by coughing, yet in the absence of any intracranial disor-der. This definition and the following diagnostic crite-ria are suggested by the Headache Classification Com-mittee of the International Headache Society (from Classification of headache disorders, cranial neuralgias and facial pains; and diagnostic criteria for primary headache disorders. Cephalalgia 1988;8[Suppl]:1–961051).
A. Is a bilateral headache of sudden onset, lasting less than 1 minute, and precipitated by coughing
B. May be prevented by avoiding coughing
C. May be diagnosed only after structural lesions such as posterior fossa tumor have been excluded by neu-roimaging
benign distal cranial arteritis See isolated benign cerebral vasculitis.
benign dystonia A congenital dystonic syndrome characterized by dystonias without accompanying fea-tures.337
benign early-onset Parkinson disease A parkinson-ian syndrome characterized by unilateral tremor and rigidity of long duration and minimal progression with onset usually in the fourth decade, sometimes follow-ing a severe febrile illness.5036
benign encephalomyelitis See postinfection fa-tigue syndrome.
benign epilepsy of children with rolandic spikes See benign childhood epilepsy with centrotemporal spikes.
102 BENIGN CHILDHOOD EPILEPSY WITH FRONTAL OR WITH MIDLINE SPIKES
benign epilepsy with occipital paroxysms See be-nign occipital epilepsy of childhood.
benign epileptic aphasia One of the benign focal (partial) epilepsies of childhood manifesting with pure intermittent speech disturbances. See benign occipital epilepsy of childhood, occipital lober epilepsy and intercalated migraine.
benign epileptiform transients of sleep (small/big/
large sharp spikes) The appearance of spike activity be-tween 5 and 135 mV recorded on the EEG uni- or bi-laterally over the anterior and middle temporal lobe re-gions of young people during non–rapid eye movement sleep stages 1 and 2, especially after a period of sleep deprivation; first described by Grey-Walter.
They are considered to have no pathological signifi-cance, but their differentiation from similar spikes as-sociated with partial epilepsy of temporal lobe origin may cause confusion.5943See also small sharp spikes.
benign episodic mydriasis Intermittent unilateral pupillary dilation, of unknown cause.1602,2388
benign essential blepharospasm See blepharo-spasm.
Benign Essential Blepharospasm/Meige Research Foundation, Inc . A nonprofit organization dedi-cated to finding the cause of and cure for ble-pharospasm, Meige disease, and related disorders. The foundation has support groups nationwide in the United States, promotes awareness and research, dis-tributes educational materials to patients and physi-cians, and serves as a referral clearing house. Address:
PO Box 12468, Beaumont, TX 77726-2468. Tel: 409-832-0788.
benign essential chorea See hereditary nonpro-gressive chorea.
benign essential tremor See essential tremor.1267 benign exertional headache Headache precipitated by any form of exercise. Subvarieties, such as weight-lifters headache and effort headache in runners,3676are recognized. This definition and the following diagnos-tic criteria are suggested by the Headache Classification Committee of the International Headache Society (from Classification of headache disorders, cranial neu-ralgias and facial pains; and diagnostic criteria for pri-mary headache disorders. Cephalalgia 1988;8[Suppl]:
1–961051).
A. Is specifically brought on by physical exercise B. Is bilateral, throbbing in nature at onset and may
de-velop migrainous features in those patients susceptible to migraine (code for migraine first)
C. Lasts from 5 minutes to 24 hours
D. Is prevented by avoiding excessive exertion, particularly in hot weather or at high altitude
D. Is not associated with any systemic or intracranial dis-order
benign familial chorea See hereditary nonprogres-sive chorea.
benign familial infantile convulsions (OMIM 601764) A dominantly inhertited epilepsy syndrome linked to chromosomes 16 and 19q11–13 and charac-terized by the occurrence of seizures, typically after the age of 4 months, over a short period. The foci are pari-eto-occipital. Paroxysmal choreoathetosis may be asso-ciated at a later age.507
benign familial macrocephaly A syndrome with the following diagnostic criteria suggested by De Myer,1384 adapted here (see Chart B–2).
Chart B–2. Criteria for the Clinical Diagnosis of Benign Familial Macrocephaly The occipitofrontal diameter is 2 standard deviations
above the mean or 98th percentile.
There is no increase in intracranial pressure (as would be shown by bulging fontanelle, palpably split sutures, or persistent vomiting.
Neurological and developmental examinations are normal.
Neurocutaneous stigmata and other somatic anomalies are absent.
There is a family history of megalencephaly.
Follow-up visits establish the normality of the patient’s developmental course, and the occipitofrontal circumfer-ence curve levels off and, although it remains high, begins to parallel the normal curve.
Radiographic findings demonstrate normal or only slightly enlarged ventricles in one of the family members with an enlarged occipitofrontal circumference, establishing that the condition is megalencephaly, not arrested hydro-cephalus or another lesion.
Chemical screening tests for metabolic disorders or lysoso-mal enzyme deficits are negative in those patients lacking some of the other criteria.
Adapted from De Myer W. Megalencephaly; types, clinical syndromes and management. Pediatr. Neurol. 1986;2:321–327, reproduced by kind per-mission from Elsevier Science.
benign familial myoclonic epilepsy See Rabot epilepsy.
benign familial neonatal convulsions (OMIM 121200) A dominantly inherited epileptic syndrome in which multiple generalized tonic-clonic seizures occur daily, starting on or after the second day of life (but al-ways in the first month), and disappearing sponta-neously within weeks, although occasional seizures may occur thereafter2926in a variant form. The physical ex-amination findings and the children’s subsequent neu-rodevelopmental progress are normal. The responsible genes have been mapped to chromosome 20q13.3 and 8q24.
Ocular, autonomic, tonic, and respiratory seizure manifestations and automatisms are also recorded.4760 benign fasciculation potential A fasciculation po-tential occurring (in retrospect) in the context of a non-progressive neuromuscular disorder. Unfortunately, its electrical characteristics do not allow confident dis-tinction from fasciculations occurring in progressive BENIGN FASCICULATION POTENTIAL 103
neuromuscular disorders, so the value of the term is questionable.
benign fasciculations and cramps (Denny-Brown and Foley syndrome) The occurrence of fasciculations in healthy people who do not have other features sug-gesting amyotrophic lateral sclerosis or of other lower motor neuron disorders such as weakness or wasting, and neither EMG nor biopsy show evidence of dener-vation.1401,4626
The fasciculations are most commonly in the leg mus-cles but can affect any region, and tend to recur in the same point of a muscle on any particular occasion. The cramps are usually felt in the calf muscles. The origin of the disorder is not known, but progression to amyo-trophic lateral sclerosis is seldom seen; only about 6%
of subjects aged 45 years and older with fasciculations as their only complaint go on to develop this disorder.
benign focal amyotrophy A sporadic syndrome of nonprogressive asymmetrical uni- or bilateral segmen-tal muscle weakness and atrophy localized to one limb or both homologous limbs markedly, occurring in young males. Sensory loss and CNS involvement do not occur. The electrophysiological, radiological, and mus-cle histopathological findings suggest chronic focal an-terior horn cell disease.3041See monomelic amyotro-phy and hereditary motor neuropathy, of which this is likely to be a variant.
benign focal atrophy See monomelic amyotrophy.
benign focal epilepsy See benign childhood epilepsy with centrotemporal spikes.
benign focal epilepsy with centrotemporal spikes See benign childhood epilepsy with centrotemporal spikes.
benign focal epilepsy with occipital lobe spike waves See occipital lobe epilepsy and intercalated migraine.
benign hereditary chorea A syndrome characterized by chorea affecting all body parts, clumsy gait, some-times with dystonia, and with onset in infancy, child-hood, or youth.4563,4732 The condition is mapped to 14q. This is surely no more than a variety of heredi-tary nonprogressive chorea.
benign hereditary myopathy See benign congenital myopathy.
benign idiopathic dystonia Dystonia occurring in the first year of life, ceasing by 18 months of age.5979 See dystonia.
benign infantile mitochondrial myopathy (mito-chondria-lipid-glycogen disease of muscle) A reversible expression of inherited deficiency in cytochrome-c ox-idase (complex IV), leading clinically to hypotonia, weakness, macroglossia, and lactic acidosis after birth, which, however, abates over months and leads only to residual mild proximal weakness.1448
benign influenzal myopathy A transient painful af-fection of the legs, with elevated serum creatine kinase levels, seen occasionally in children recovering from acute influenzal infections.3868
benign intracranial hypertension One of two ac-ceptable alternative terms for the syndrome of in-creased intracranial pressure in the absence of a causative mass lesion, documented elsewhere in this book under idiopathic intracranial hypertension.
benign intracranial hypertension headache The following diagnostic criteria are suggested by the Headache Classification Committee of the Interna-tional Headache Society (from Classification of headache disorders, cranial neuralgias and facial pains;
and diagnostic criteria for primary headache disorders.
Cephalalgia 1988;8[Suppl]:1–961051).
A. Patient suffers from benign intracranial hypertension fulfilling the following criteria: increased intracranial pressure (200 mm H2O) measured by epidural or in-traventricular pressure monitoring or by lumbar punc-ture; normal neurological examination except for pa-pilledema and possibly sixth cranial nerve palsy; no mass lesion and no ventricular enlargement on neuroimag-ing; normal or low protein concentration and normal white blood cell count in CSF; no clinical nor neu-roimaging suspicion of venous sinus thrombosis.
B. Headache intensity and frequency related to variations of intracranial pressure with a time lag of less than 24 hours.
benign juvenile focal muscular atrophy of upper ex-tremities See monomelic amyotrophy.
benign masturbatory cephalalgia An acute benign exertional headache, usually experienced in pri-vate.5768See headache associated with sexual activity.
benign monoclonal gammopathy See parapro-teinemic neuropathy.
benign monomelic amyotrophy See monomelic amyotrophy.
benign motor neuron disorder See cramp fascicu-lation syndrome.
benign multiple sclerosis That form of multiple sclerosis which, having been present for more than 10 years with a relapsing and remitting course, has left the patient with little functional disability (rating less than 3 on the Kurtzke scale).
benign muscular dystrophy with contractures A variant of Emery-Dreifuss syndrome with likely auto-somal dominant inheritance, characterized by proximal myopathy, contractures, and rigidity of paraspinal muscles without cardiomyopathy.295
A dominant pattern of inheritance is likely, but Y-to-Y transmission is also possible.295
benign myalgic encephalomyelitis See chronic fa-tigue syndrome.
104 BENIGN FASCICULATIONS AND CRAMPS